Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
Users online: 11758  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page

Table of Contents 
Year : 2016  |  Volume : 61  |  Issue : 2  |  Page : 237
Acrokeratosis verruciformis of hopf clinically mimicking epidermodysplasia verruciformis

Department of Dermatology and Venereology, Pramukhswami Medical College, Karamsad, Gujarat, India

Date of Web Publication1-Mar-2016

Correspondence Address:
Nilofar G Diwan
Department of Dermatology and Venereology, Pramukhswami Medical College, Karamsad, Gujarat
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.177783

Rights and Permissions

How to cite this article:
Diwan NG, Jivani NB, Nair PA. Acrokeratosis verruciformis of hopf clinically mimicking epidermodysplasia verruciformis. Indian J Dermatol 2016;61:237

How to cite this URL:
Diwan NG, Jivani NB, Nair PA. Acrokeratosis verruciformis of hopf clinically mimicking epidermodysplasia verruciformis. Indian J Dermatol [serial online] 2016 [cited 2023 Sep 25];61:237. Available from:


A 34-year-old female presented to skin outpatient department with multiple asymptomatic discrete hyperpigmented and hypopigmented lesions over scalp, face, neck, trunk, and both limbs since 2 years. No specific family history present. On examination, multiple hyperpigmented macules and papules were present over neck, back, face [Figure 1]a, both upper and lower limbs and scalp [Figure 1]b. Multiple hypopigmented macules were present over back, chest, and limbs [Figure 2]a and [Figure 2]b. Woods lamp examination of hypopigmented lesions did not revealed any fluroscence or accentuation. No orogenital involvement and nail changes noted. Biopsy was taken from hyperpigmented papule keeping epidermodysplasia verruciformis (EDV), Darier's disease and acrokeratosis verruciformis of Hopf (AKV) as differentials. Other biopsy was taken from hypopigented lesion with tinea versicolor and EDV as differential. Biopsy from both sites showed epidermis with hyperkeratosis, moderate acanthosis and papillomatosis. Rete ridges were elongated with circumscribed elevation of epidermis giving characteristic church spire appearance [Figure 3]a and [Figure 3]b and mild perivascular lymphocytic infiltrates in upper dermis favoring AKV. Patient was advised topical retinoids over hyper pigmented lesions. Clinical improvement was noted in the form of reduced pigmentation after a month of follow-up.
Figure 1: Multiple hyperpigmented macules and papules over (a) neck, chest and face (b) scalp

Click here to view
Figure 2: Multiple hypopigmented macules over (a) chest (b) lower limbs

Click here to view
Figure 3: (a and b) Hyperkeratosis, moderate acanthosis and papillomatosis with elongated rete ridge and circumscribed elevation of epidermis giving characteristic church spire appearance (H and E, ×4 and ×10)

Click here to view

AKV is a rare genodermatosis, which develops at birth or during early childhood, but the onset may be delayed up to the fifth decade. It is more common in males as compared to females with a ratio of 5:1.3.[1] It results from a single dominant genetic defect with variable expressivity and is an allelic disorder arising from a mutation in ATP2A2.[2] Clinically, it presents as dry, rough, skin-colored or reddish-brown, flat-topped, or warty papules resembling flat warts on the dorsum of the hands and feet, on the knees, elbows, forearms, or lower legs. Forehead, scalp, flexures, and oral mucosa are never affected, as reported by Panja.[3] but scalp, forehead, neck, and upper trunk were mainly affected in our case. The palmar skin may be thickened and show punctate keratosis, pits, or punctiform breaks in dermatoglyphics. Nail involvement occurs in the form of longitudinal splitting, striations, and subungual hyperkeratosis.[4]

Classical AKV often occurs during childhood whereas the onset age of sporadic AKV is much later than that of classical AKV. Clinically typical morphology is seen at dorsum of hands and feet in classical variant while in sporadic it can affect other sites like face, scalp, trunk rather than classical sites as seen in our case. Positive family history with palmar pits and nail changes are seen in classical AKV but not in sporadic AKV.[5]

EDV is a rare autosomal recessive condition, clinically characterized by a combination of pityriasis Versicolor like hypopigmented lesions, verruca plana-like lesions, and seborrheic keratosis-like verrucous plaques.[6]

All the findings in our case were in favor of sporadic AKV with late age of onset, lesions over face, scalp, and trunk, without the involvement of palms, nails and insignificant family history. Such cases are reported but are uncommon.[7] Histopathologically, AKV shows exaggeration of the epidermal layer and thickening of the granular layer. These layers show a blunt, domed pattern within concavities of the wavy stratum corneum, so called “church spire.” There can be some degree of acantholysis, but not parakeratosis, dyskeratosis, or basal layer change.[3] Darier's disease, EDV, plane warts, and seborrheic keratoses, clinically simulate lesions of AKV, but they can be distinguished on the basis of histologic features. Hyperkeratosis and hypergranulosis with vacuolation and koiliocytes with mild to moderate dysplasia in the epidermis are consistent with the diagnosis of EDV while the absence of squamous and basaloid cells rules out the possibility of seborrheic keratosis. The only effective treatment of AKV is superficial ablation. Our case was unique with a sporadic variant of AKV in a female gender having onset age later in life, absent family history, no palmar pits or nail changes and involvement of neck, face, and scalp. Hypopigmented macules were also unique over trunk and extremity mimicking EDV.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Kaliyadan F, Manoj J, Venkitakrishnan S. Acrokeratosis verruciformis of Hopf associated with dilated cardiomyopathy. Indian J Dermatol 2009;54:296-7.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
Berk DR, Taube JM, Bruckner AL, Lane AT. A sporadic patient with acrokeratosis verruciformis of Hopf and a novel ATP2A2 mutation. Br J Dermatol 2010;163:653-4.  Back to cited text no. 2
Panja RK. Acrokeratosis verruciformis: (Hopf) – A clinical entity? Br J Dermatol 1977;96:643-52.  Back to cited text no. 3
Rallis E, Economidi A, Papadakis P, Verros C. Acrokeratosis verruciformis of Hopf (Hopf disease): Case report and review of the literature. Dermatol Online J 2005;11:10.  Back to cited text no. 4
Bang CH, Kim HS, Park YM, Kim HO, Lee JY. Non-familial acrokeratosis verruciformis of Hopf. Ann Dermatol 2011;23 Suppl 1:S61-3.  Back to cited text no. 5
Sterling JC. Virus infections. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 7th ed. West Sussex: Wiley Blackwell Publishers; 2004. p. 25.58-9.  Back to cited text no. 6
Mishra DK, Singh AK. Acrokeratosis verruciformis of Hopf. Indian J Dermatol Venereol Leprol 1995;61:357.  Back to cited text no. 7
[PUBMED]  Medknow Journal  


  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
1 Acrokeratosis verruciformis of hopf: An unusual presentation
K Lohanee, DP Thapa
Indian Dermatology Online Journal. 2021; 12(6): 928
[Pubmed] | [DOI]


Print this article  Email this article
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (816 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

    Article Figures

 Article Access Statistics
    PDF Downloaded70    
    Comments [Add]    
    Cited by others 1    

Recommend this journal